Disease Directory Behcet Disease
Immune

Behcet Disease

Also known as: Behcet syndrome, Behcet's disease, triple symptom complex, silk road disease

Prevalence

1-10 in 100,000 in Western countries; up to 420 in 100,000 in Turkey

Onset

Young adulthood (20s-30s), rarely pediatric

Type

Variable vessel vasculitis / autoinflammatory syndrome

About Behcet Disease

Behcet Disease is a systemic vasculitis of unknown etiology involving vessels of all sizes and characterized by the pathognomonic triad of recurrent oral aphthous ulcers, genital ulcers, and ocular inflammation, with additional potential involvement of the skin, joints, gastrointestinal tract, and central nervous system. Strong HLA-B51 association suggests an autoinflammatory mechanism, though the precise immunopathogenesis remains incompletely understood. The disease follows a relapsing-remitting course and can cause irreversible organ damage, particularly vision loss from uveitis and neurological deficits from neuro-Behcet.

Common Clinical Features

Recurrent painful oral aphthous ulcers (≥3 episodes per year) Recurrent genital ulcers with scarring Uveitis (anterior, posterior, or panuveitis) Pathergy reaction (skin hyper-reactivity to trauma) Erythema nodosum and pseudofolliculitis Thrombophlebitis and deep vein thrombosis Neuro-Behcet (headache, brainstem lesions, cognitive change)

Clinical Trial Eligibility Tips

What to know before applying to Behcet Disease trials.

International Study Group diagnostic criteria fulfillment is typically required; compile documentation of ulcer frequency, eye exam reports, and pathergy test results

Uveitis trials often stratify by ocular involvement severity and prior biologic use (anti-TNF, interferon); have complete ophthalmology records and visual acuity measurements available

Active major vessel disease (arterial aneurysm, venous thrombosis) may require separate anticoagulation management and can affect trial eligibility — disclose vascular history fully

Patient Resources

Patient Organization

American Behcet's Disease Association

Visit website ↗

Natural History Registry

International Behcet Registry

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:117)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting Behcet Disease trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Behcet Disease. Updated daily.

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